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Giuliano Binetti, MD; Joseph J. Locascio, PhD; Suzanne Corkin, PhD; Jean Paul Vonsattel, MD; John H. Growdon, MD

Arch Neurol. 2000;57:225-232.

Objectives  To define the cognitive characteristics of Pick disease (PcD), and to determine which features distinguish PcD from Alzheimer disease (AD), in a cross-sectional and longitudinal study.

Methods  The participants were 44 patients with PcD (10 pathologically verified), 121 patients with AD (14 pathologically verified), and 60 normal control subjects. We obtained information regarding the initial symptom of dementia from each patient's caregiver, estimated global dementia severity by the Blessed Dementia Scale and the Activities of Daily Living Scale, and assessed specific cognitive domains by administering 10 tests of memory, language, visuospatial, and reasoning abilities and selective attention.

Results  Among initial symptoms reported by caregivers, personality change and language impairment were significantly more common in PcD than AD; deficits in memory were common in both groups but more prevalent in AD (P<.001). At initial cognitive testing, the scores of patients with PcD were inferior to those of normal controls on all tests, except on a measure of visuospatial function; the scores of patients with AD were inferior to those of controls on all tests. Patients with PcD were superior to patients with AD on measures of explicit memory (P<.001) and visuospatial function (P = .001) but had greater impairments on the Activities of Daily Living Scale (P<.05). During the course of illness, patients with PcD declined significantly faster than those with AD on language tests and on global measures of dementia severity (P<.05), whereas measures of explicit memory and visuospatial and reasoning abilities worsened equally in both patient groups.

Conclusions  There is a characteristic cognitive profile and course of dementia in PcD. Nonetheless, cognitive test performance does not clearly distinguish PcD from AD.

From the Department of Brain and Cognitive Sciences and the Clinical Research Center, Massachusetts Institute of Technology, Cambridge (Drs Binetti, Locascio, and Corkin); Department of Neurology, Massachusetts General Hospital, Boston (Drs Binetti, Locascio, and Growdon); Department of Neuropathology, Laboratory for Molecular Neuropathology, Massachusetts General Hospital, Charlestown (Dr Vonsattel); and the Alzheimer's Disease Research Unit, Istituto di Ricovero e Cura a Carattere Scientifico, S Giovanni di Dio, Brescia, Italy (Dr Binetti).

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